Abstract

Life expectancy of cystic fibrosis (CF) patients increased up to 30 years in developed countries with the new treatment modalities. There are no clear data about the incidence of CF in Turkey and there are few adult CF patients. In this study; clinical characteristics and treatment modalities of 15 adolescent and adult CF patients who had been followed at Marmara University, Division of Pediatric Pulmonology are reported. CF was diagnosed with a positive sweat test (sweat Chloride ³ 60 mEq/L) in the presence of clinical symptoms. Mean age of our patients is now 18.9±4.8 (15-28) years and these patients had been followed in our clinic for a mean duration eksof 4.6±3.0 (1-12) years. Although the symptoms started at the age of 2.3±3.7 (0-13) years, mean age of diagnosis was 11.3±7.1 (0.2-27) years. Presenting symptoms were cough (93%), wheezing (14.3%) and sputum (66.7%). There was consanguinity in 20% and history of sibling deaths in 33.3% of our patients. Pulmonary function tests revealed mean FEV1 of 57.7±27%predicted, and mean FVC of 64.1±28.3. Based on pulmonary function tests, patients were classified as severe (26% had FEV1<40%), moderate (33% had FEV1 of 41%- 69%), and mild (41% had FEV1 >70%). Sputum cultures were positive for S. aureus in 13.3% (n=2) and for P. aeruginosa in 60% (n=9) of patients. Liver enzyme levels were persistently elevated in 20% (n=5). Nebulized DNAse (Pulmozyme“) was used to reduce the sputum viscosity in 53.3% (n=8) and nebulized tobramycin was used for patients who were colonized with P. aeruginosa (60% (n=9). Pancreatic insufficiency was present in 80% (n=12) of patients who used Kreon for pancreatic enzyme replacement. One patient died during follow up, all other patients are still followed. In conclusion, although these patients had symptoms starting at the early ages, CF was diagnosed 8 years after the symptoms started. Since the early diagnosis and treatment reduces mortality and morbidity of CF, pediatricians and adult physicians in our country should be educated in diagnosing and treating this disease.